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Scleroderma Renal Crisis — RACP Adult Medicine MCQ

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HardRheumatologyScleroderma Renal CrisisRACP Adult Medicine

A 45-year-old woman with systemic sclerosis develops sudden onset severe hypertension (BP 240/140 mmHg), headache, visual disturbance, and acute kidney injury (creatinine rising from 80 to 400 µmol/L in 48 hours). Blood film shows schistocytes. What is the critical first-line treatment?

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Correct answer: A — ACE inhibitor (captopril – short-acting, titrated to response)

This is scleroderma renal crisis (SRC) – a medical emergency characterised by malignant hypertension and rapidly progressive renal failure, often with microangiopathic haemolytic anaemia. ACE inhibitors are the CRITICAL first-line treatment – captopril (short-acting, easy to titrate) is preferred, starting at 6.25–12.5 mg and titrating rapidly to control BP. ACE inhibitors have transformed the prognosis of SRC from near-universal mortality to survival in >70%. They should be continued even if dialysis is required.

Reference: eTG – 2025 – Rheumatology; EULAR – 2023 – SSc Guidelines